1/6. Intraductal papillary mucinous tumors: a case report and review of literature. Intraductal papillary mucinous tumors are slow growing cystic neoplasms of pancreas with favorable prognosis. A case report of such a tumor in an octogenarian is presented. Excessive mucin secretion with ectatic dilatation of pancreatic ducts is a hallmark of this tumor. K-ras and p53 mutations are less frequent then in ductal pancreatic adenocarcinoma. Endoscopic retrograde cholangiopancreatography findings are diagnostic and include a patulous papilla and amorphous filling defects in dilated pancreatic ducts. Magnetic resonance pancreatography, endoluminal ultra-sound, and pancreatoscopy are also helpful in diagnosis. Complete surgical resection is recommended. ( info) |
2/6. Mucinous cystic lesions of the pancreas. Report of a patient submitted to distal pancreatectomy. Intraductal papillary neoplasm consists a variant of the mucin-producing neoplasms of the pancreas; these are rare diseases that can be divided into mucinous ductal ectasias and intraductal papillary neoplasms. Such neoplasms have a malignant potential, and their prognosis is good if they are early diagnosed and localized surgical resection. This paper describes a case of an intraductal papillary neoplasm localized in the tail of the pancreas and treated by distal splenopancreatectomy. The literature on this rare disease is also reviewed. ( info) |
| Cystic sebaceous neoplasms have been seen only in patients with muir-torre syndrome (MTS) and have recently been characterized as marker lesions of MTS. Histologically, these lesions form a spectrum of tumors ranging from benign cystic adenomas to proliferative cystic sebaceous tumors. We describe 2 proliferative cystic sebaceous tumors in a 53-year-old man whose workup revealed colonic adenocarcinoma and other sebaceous tumors consistent with MTS. Both the chest wall and the left thigh masses were grossly cystic, measuring 1.0 and 1.5 cm, respectively. Histologic sections demonstrated well-circumscribed cystic neoplasms located in the deep dermis and subcutaneous tissue. Each had a focally infolded cyst wall composed of immature basaloid cells with prominent nucleoli and mitoses, consistent with a proliferative cystic sebaceous tumor. Recognition of cystic sebaceous neoplasm by pathologists and communication to clinicians of its strong association with MTS is of diagnostic importance. ( info) |
4/6. Major hepatectomy and right hemicolectomy at the time of primary cytoreductive surgery for advanced ovarian cancer: report of a case. Major liver involvement at the time of diagnosis is a rare event in patients with ovarian cancer, and the issue of major hepatectomy at the time of primary cytoreductive surgery is controversial. A 61-year-old woman was admitted to our hospital with nonspecific abdominal pain of 2-month duration and weight loss of 5 kg during the last semester. A computed tomography scan demonstrated bilateral ovarian masses, extending to the right iliac fossa, pressing the cecum-ascending colon. In the liver parenchyma, three cystic lesions were found of about 6-cm maximum diameter each, along with pelvic lymphadenopathy. There was no ascites. The diagnosis of advanced ovarian cancer was clinically suspected; the patient underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy, right hemicolectomy, omentectomy, left lobectomy, deroofing, and draining of the cystic formation of the right liver lobe along with systematic pelvic and para-aortic lymphadenectomy. Systemic chemotherapy (six cycles of paclitaxel/carboplatin) was subsequently administered, and after 15 months of follow-up period, the patient is still in first remission and alive. Ovarian cancer with concomitant extensive right colon infiltration and hematogenous liver metastases can be successfully managed with aggressive surgical resection and postoperative chemotherapy in carefully selected patients. ( info) |
5/6. Obstructive chronic pancreatitis and/or intraductal papillary mucinous neoplasms (IPMNs): a 21-year long case report. CONTEXT: Intraductal papillary mucinous neoplasms of the pancreas (IPMNs), usually present with a picture of chronic pancreatitis and must be considered within the differential diagnosis of this condition. CASE REPORT: We report a long history of a 58-year-old male who presented with recurrent attacks of pancreatitis. He was treated for chronic pancreatitis until the age of 78 when the diagnosis of IPMN was made, but radical treatment was not possible and he was managed palliatively. The patient died of sepsis and multi-organ failure 21 years following his first presentation. CONCLUSION: This case highlights possible pitfalls in the diagnosis and treatment of IPMNs, suggests a very long natural history, proposes possible palliative treatment in selected cases and reflects about IPMNs biological position in the chronic pancreatitis-ductal adenocarcinoma chain. ( info) |
6/6. Cystic pulmonary metastases of endometrial stromal sarcoma of the uterus, mimicking lymphangiomyomatosis: a case report with immunohistochemistry of HMB45. A case of endometrial stromal sarcoma (ESS) showed cystic pulmonary metastases mimicking lymphangiomyomatosis (LAM). A 58-year-old female, who had undergone total hysterectomy for low-grade ESS 16 years previously, had repeated bouts of pneumothorax. Multiple thin-walled cysts in the peripheral lung were revealed by radiological examinations. In an open-lung biopsy specimen, cystic lesions were surrounded by layers of spindle-shaped cells of varying thickness that resembled LAM. However, in addition to subtle histologic differences from LAM, HMB45 (antimelanoma antibody) showed positive in LAM (n = 3), but was negative in ESS (n = 2) and the cystic lesions of this case. Using myogenic markers (desmin and alpha-smooth muscle actin), metastatic ESS could be immunohistochemically differentiated from mesenchymal cystic hamartoma (n = 1). HMB45 immunohistochemistry is useful in the differential diagnosis of cystic pulmonary lesions. ( info) |